RESUMEN
We investigated the clinicopathological features and prognostic factors of patients with peripheral T-cell lymphoma (PTCL) in 13 sites across Spain. Relevant clinical antecedents, CD30 expression and staining pattern, prognostic indices using the International Prognostic Index and the Intergruppo Italiano Linfomi system, treatments, and clinical outcomes were examined. A sizeable proportion of 175 patients had a history of immune-related disorders (autoimmune 16%, viral infections 17%, chemo/radiotherapy-treated carcinomas 19%). The median progression-free survival (PFS) and overall survival (OS) were 7·9 and 15·8 months, respectively. Prognostic indices influenced PFS and OS, with a higher number of adverse factors resulting in shorter survival (P < 0·001). Complete response (CR) to treatment was associated with better PFS (62·6 vs. 4 months; P < 0·001) and longer OS (67·0 vs. 7·3 months; P < 0·001) compared to no CR. CD30 was expressed across all subtypes; >15% of cells were positive in anaplastic lymphoma kinase-positive and -negative anaplastic large-cell lymphoma and extranodal natural killer PTCL groups. We observed PTCL distribution across subtypes based on haematopathological re-evaluation. Poor prognosis, effect of specific prognostic indices, relevance of histopathological sub-classification, and response level to first-line treatment on outcomes were confirmed. Immune disorders amongst patients require further examination involving genetic studies and identification of associated immunosuppressive factors.
Asunto(s)
Linfoma de Células T Periférico/patología , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Femenino , Humanos , Antígeno Ki-1/análisis , Linfoma de Células T Periférico/diagnóstico , Linfoma de Células T Periférico/epidemiología , Masculino , Persona de Mediana Edad , Pronóstico , Estudios Retrospectivos , España/epidemiología , Análisis de Supervivencia , Adulto JovenAsunto(s)
Adenocarcinoma/metabolismo , Regulación Neoplásica de la Expresión Génica , Neoplasias Pulmonares/metabolismo , MAP Quinasa Quinasa 5/metabolismo , Proteína Quinasa 7 Activada por Mitógenos/metabolismo , Animales , Línea Celular Tumoral , Femenino , Perfilación de la Expresión Génica , Humanos , Inmunohistoquímica , Estimación de Kaplan-Meier , Masculino , Ratones , Ratones Transgénicos , Trasplante de NeoplasiasRESUMEN
Autoimmune enteropathy (AIE) is an infrequent cause of malabsorption that is usually associated with the presence of circulating autoantibodies and a predisposition to autoimmune disorders. This disease is more frequent in children. The diagnosis of this disorder is based on five criteria: chronic diarrhea (>6 weeks), malabsorption, specific histological findings, exclusion of similar disorders, and the presence of specific antibodies such as anti-enterocyte and anti-goblet cell antibodies. We present the case of a 63-year-old patient with chronic diarrhea, weight loss and significant deterioration of nutritional status.
